Background Chordomas of the upper cervical spine are rare and present unique surgical challenge.This study aimed to describe the clinical characteristics and surgical management of patients with chordomas of the upper cervical spine.Methods Twenty-one patients with chordomas of the upper cervical spine who were treated in Peking University Third Hospital from January 1999 to October 2012 were retrospectively analyzed.Survival was calculated by the Kaplan-Meier method and was compared between groups using the log-rank test.Results The postoperative diagnosis was classical chordoma in 20 cases and chondroid chordoma in one case.The mean operative time was 9.5 hours (range 6-17 hours),and the mean blood loss was 2 812 ml (range 700-4 800 ml).There were two postoperative deaths.Unilateral vertebral artery ligation was performed in six patients,cervical nerve roots were cut in six patients,and the extal branch of the superior laryngeal nerve was repaired after being cut in one case.Two patients developed postoperative velopharyngeal incompetence,and loosening of the occipitocervical screws was observed in one patient.The recurrence rate was 66.7％ (10/15) after a mean follow-up period of 46.8 months (range 14-150 months).The 5-and 10-year overall survival rates were (39.8±13.1)％ and (31.9±12.7)％,respectively.There was a significant difference in survival rate between patients who underwent surgery and those who did not.Conclusion In spite of the high rates of recurrence and complications after surgical treatment of chordomas of the upper cervical spine,intralesional resection combined with adjuvant radiotherapy remains the optimal treatment to prolong survival.